Infantile amaurotic idiocy (Tay-Sachs disease | vedclass

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(B) Tay-Sachs disease is the most common $GM_2$ gangliosidosis,occurring almost exclusively among north-east European Jews.It is characterized by infa

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An inherited disorder in which the spinal cord and brain are severely damaged within a few months of birth leading to paralysis and mental retardation,and finally the infant's death in $3$ or $4$ years

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(B) Tay-Sachs disease is the most common $GM_2$ gangliosidosis,occurring almost exclusively among north-east European Jews.It is characterized by infantile onset ($3-6$ months),doll-like facies,cherry-red macular spot ($90+$ percent of the infants),early blindness,hyperacusis,macrocephaly,seizures,and hypotonia.The children typically die between $2$ and $5$ years of age.It is caused by the deficiency of the $hexosaminidase A$ enzyme in tissues.

Infantile amaurotic idiocy $(Tay-Sachs disease)$ is

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